AB1239 PRIMARY SJÖGREN’S SYNDROME WITH JUVENILE ONSET: REPORT OF 15 CASES
Authors: M. Kaleda, I. Nikishina, S. Salugina, E. Fedorov, A. Shapovalenko, T. Pachkoria, S. Arsenyeva
Journal: Annals of the Rheumatic Diseases (2022)

Background Primary Sjögren’s syndrome (pSS) is a chronic and progressive multisystem autoimmune disease which rarely onset in children and adolescents. Diagnostic delay in large part of patients are common due to the non-specific and variable symptoms and the slow progression of disease. Objectives To analyse demographic data, specific extraglandular, salivary and ocular manifestations, laboratory parameters and therapy of pSS with juvenile onset. Methods Retrospective study of all patients (pts) with pSS in single center. Results pSS was verified in 15 pts (6.7% were boys), which amounted to 23.8% of all pts with SS in our pediatric rheumatologic department. The median age of pSS onset was 8.0 y.o. [IQR 7.0; 10.2]. The median of disease duration at the time of pSS verification was 2.75 years [2.2; 5.6]. All patients had systemic manifestations at onset: constitutional abnormalities – 33.3%, nonerosive polyarthritis – 64.3%, polyarthralgias – 26.7%, lymphadenopathy – 73.3%, cutaneous involvement – 53.3% (2 – xerosis, 2 - annular erythema, 1 - erythema nodosum, 2 – Raynaud phenomenon, 2 – nonspecific spotty rashes, 1 – hemorrhagic rash). At the time of diagnosis 7 pts (46.7%) had isolated involvement of salivary glands, 8 pts (53.3%) – combined with involvement of lacrimal glands. The decrease in salivary gland function was recorded in 80% of cases, hypolacrimia – in 46.7%, 1 patient had isolated hypolacrimia. Recurrent parotitis was present in 6 pts (40.0%). At time of diagnosis pulmonary involvement had 20.0% of pts, 1 patient had renal tubular acidosis. 8 pts (53.3%) had various hematological disorders: anemia – in 3 pts (20.0%), leukopenia – in 6 (40.0%). ANA Hep-2 were detected in 100% pts (in titer 1/640 – 4, 1/1280 – 7, 1/2560 – 3, 1/20480 - 1, with mixed patterns in all pts: speckled + homogeneous – 9 pts, speckled + homogeneous+cytoplasmic – 6 pts), anti-Ro - in 12 pts (80.0%), anti-La - in 8 pts (53.3%), RF+ - in 9 pts (60.0%). 6 pts (40.0%) had polyclonal hypergammaglobulinemia, max 42%. 2 pts (13.3%) had concomitant autoimmune non-rheumatic disease; 1 – cutaneous psoriasis, 1 - autoimmune thyroiditis. The treatment of each patient was justified by the main individual manifestations: 93.3% received glucocorticoids, 26.7% - methotrexate, 33.3% - hydroxychloroquine, 6.7% - mycophenolate mofetil. Treatment with biologics (B) was received by 13 (93.3%) pts (7 – rituximab (RTM), 6 – abatacept (ABA)) with a good response in 10 pts, including improvement in the function of the salivary and lacrimal glands in 7 pts. 1 patient received 2B - RTM and ABA sequentially due to the development of MAS 7 days after 1 st RTM infusion. B was discontinued in 3 pts: 1 due to development of hemorrhagic vasculitis 2 days after the 1 st RTM infusion, 1 – COVID-19 with lung involvement (CT 3-4) 2 weeks after the 1st RTM infusion, 1 – inefficiency of ABA during 15 months. Conclusion In our pediatric rheumatologic department pts with pSS made up less than a quarter of all pts with SS. The diagnosis was verified delayed in all pts, which can be explained by a wide range of nonspecific manifestations at the onset. However, the manifestations of SS that were present at the time of diagnosis were brought under control on the background of complex therapy, including the prescription of B, with a good efficacy and safety profile of therapy. Disclosure of Interests None declared